• 제목/요약/키워드: Neuroendocrine neoplasms

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Clinical Management of Gastric Neuroendocrine Tumors

  • In Kyung Yoo;Bora Keum
    • Journal of Digestive Cancer Research
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    • 제3권1호
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    • pp.21-25
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    • 2015
  • Gastric neuroendocrine tumors (GNETs, also known as gastric carcinoids) are rare form of hormone-secreting neoplasms that present with varied clinical syndromes. There are four types of GNETs based on size, proliferation, localization, differentiation, and hormone production. Type I GNET is related to autoimmune atrophic gastritis and hypergastrinemia. Type II GNETs are related to multiple endocrine neoplasia (MEN)-1, Zollinger-Ellison syndrome and hypergastrinemia. Type 3 GNETs are not associated with any background pathology, and type 4 GNETs are poorly differentiated tumors. The most useful diagnostic and prognostic marker for gastrointestinal NETs is plasma chromogranin A (CgA) levels. Endoscopic ultrasound is the method of choice to determine tumor size and depth of infiltration. For optimal management, the type, biology, and stage of the tumor must be considered. Here, we provide a comprehensive and up-to-date review of GNETs.

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Digestive Neuroendocrine Tumor Distribution and Characteristics According to the 2010 WHO Classification: a Single Institution Experience in Lebanon

  • Kourie, Hampig Raphael;Ghorra, Claude;Rassy, Marc;Kesserouani, Carole;Kattan, Joseph
    • Asian Pacific Journal of Cancer Prevention
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    • 제17권5호
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    • pp.2679-2681
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    • 2016
  • Background: Gastro-entero-pancreatic neuroendocrine neoplasms (GEP-NEN) are relatively rare tumors, not equally distributed in gastro-intestinal system. In 2010, a revised version of the WHO classification of GEP-NENs was published. This study reports for the first time the distribution and characteristics of GEP-NEN in a Lebanese population. Materials and Methods: This descriptive retrospective study concerns all the digestive neuroendocrine tumors with their characteristics diagnosed in $H\hat{o}tel$ Dieu de France in Beirut, Lebanon from 2001 to 2012, all the pathology reports being reanalyzed according to the latest WHO 2010 classification. The characteristics and features of GEP-NEN analyzed in this study were age, gender, grade and site. Results: A total of 89 GEP-NENs were diagnosed, representing 28.2% of all neuroendocrine tumors. The mean age of GEP-NEN patients was 58.7 years and the M/F sex ratio was 1.2. The primary localization was as follows: 21.3%(19) pancreatic, 18% (16) gastric, 15.7% (14) duodenal, 11.2% (10) appendix, 10.1% (9) intestinal, 10.1% (9) colorectal (7.9% colonic and 2.2% rectal), 5.6% (4) hepatic, 2.2% (2) ampulla, 1.1% (1) esophageal and 7.9%(5) NOS digestive (metastatic with unknown primary). Of the 89 patients with GEP-NEN, 56.2% (50) were diagnosed as grade I, 11.2% (10) as grade II, 20.2% (18) as grade III and 12.4% (11) were considered as mixed adeno-neuroendocrine carcinomas (MANEC). Conclusions: This study, one of the rare examples based on the 2010 WHO classification of neuroendocrine tumors in the literature, indicates that in the Lebanese population, all duodenal and appendicular tumors are G1 and the majority of MANEC tumors are gastric and pancreatic tumors. Moreover, more duodenal tumors and fewer rectal tumors were encountered in our study compared to European reports.

Mixed Exocrine and Endocrine Carcinoma in the Stomach: A Case Report

  • Lee, Han-Hong;Jung, Chan-Kwon;Jung, Eun-Sun;Song, Kyo-Young;Jeon, Hae-Myung;Park, Cho-Hyun
    • Journal of Gastric Cancer
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    • 제11권2호
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    • pp.122-125
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    • 2011
  • We report a rare case of the coexistence of a gastric small cell neuroendocrine carcinoma with a gastric adenocarcinoma. A 62-year-old man presented with epigastric soreness for 1 month. Esophagogastroduodenoscopy revealed a Borrmann type I tumor at the lesser curvature of the lower body of the stomach. The patient underwent a distal gastrectomy with D2 lymph node dissection and the resected specimen exhibited a $3.5{\times}3.5$ cm sized, fungating lesion. Two separated, not intermingling, lesions with non-adenocarcinoma components encircled by well differentiated adenocarcinoma components were identified microscopically. The non-adenocarcinoma component showed neuroendocrine features, such as a solid and trabecular pattern, and the tumor cells showed a high nuclear grade with minimal cytoplasm, indistinct nucleoli, and positive response for synaptophysin, CD56. The final pathological diagnosis was a gastric mixed exocrine-endocrine carcinoma (MEEC) composed of an adenocarcinoma and small cell neuroendocrine carcinoma of the collision type.

A Case of Pancreatic Neuroendocrine Tumor Accompanied by a Cystic Change in Early Stage

  • Sang Soo Bae;Eun Jeong Kim;Dong Wook Lee;Ho Gak Kim;Jimin Han
    • Journal of Digestive Cancer Research
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    • 제5권1호
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    • pp.50-54
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    • 2017
  • Pancreatic neuroendocrine tumors are rare pancreatic neoplasms comprising 1-2% of all pancreatic tumors and typically present high attenuating mass on arterial and venous phase images, due to their rich capillary network. A 70-year-old South Korean female visited our hospital presenting with jaundice and dark urine color. She had received an operation for treatment of small bowel perforation seven years ago. On physical examination, icteric sclera was observed but otherwise unremarkable. Laboratory tests were abnormal liver function test and suspected obstructive jaundice. Computed tomography revealed 4 cm sized cystic mass lesion with homogeneous low attenuation in the head of pancreas and distal common bile duct was compressed by the mass. During review of past medical records, we found that the mass was observed and measured about 1.7 cm seven years ago. To resolve obstructive jaundice, pylorus preserving pancreaticoduodenectomy was performed and diagnosed with well differentiated pancreatic neuroendocrine carcinoma with intermediate grade.

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크기가 작은 췌장 신경내분비종양의 관리 (Management of Small Pancreatic Neuroendocrine Neoplasm)

  • 백우현;이경주;장성일;조재희
    • Journal of Digestive Cancer Research
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    • 제9권1호
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    • pp.19-24
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    • 2021
  • 고해상도 영상기법과 내시경술기의 발전으로 인해 작고 무증상인 췌장 신경내분비종양의 발생률은 증가하고 있다. 대부분의 췌장 신경내분비종양은 느리게 성장하나 때때로 예후가 좋지 않은 국소 침윤 또는 전이를 보인다. 작고 비기능성인 췌장 신경내분비종양의 관리 방법에 대해서는 아직 정립되지 않았다. 미국종합암네트워크 지침은 2 cm 미만의 작은 췌장 신경내분비종양에서는 관찰을 권장한다. 외과 의사의 의견은 두 부류로 나뉘어진다. 작은 췌장 신경내분비종양에서도 악성의 위험이 높으므로 공격적인 수술 치료가 필요하다는 "매"파와 악성의 위험이 있지만 과도하게 치료할 위험이 훨씬 더 높다고 주장하는 "비둘기"파가 있다. 췌장수술은 28-30%의 이환율과 1%의 사망률로 고위험 수술이기 때문에 작은 췌장 신경내분비종양에 대한 치료 결정은 어렵다.

Neuroendocrine tumors in the Iran Cancer Institute: Predictive Factors of Patient Survival

  • Sadighi, Sanambar;Roshanaee, Ghodratollah;Vahedi, Saba;Jahanzad, Easa;Mohagheghi, Mohammad Ali;Mousavi-Jarahi, Alireza
    • Asian Pacific Journal of Cancer Prevention
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    • 제15권18호
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    • pp.7835-7838
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    • 2014
  • Background: Neuroendocrine tumors have widespread and different clinical presentations and prognoses. This study was conducted to assess their survival time and prognostic factors in Iran. Materials and Methods: In a retrospective cohort study, 189 patients diagnosed of having neuroendocrine carcinoma were chosen. The tumor and clinical characteristics of the patients were modeled with a Cox proportional hazard approach. Survival was assessed using Kaplan-Meyer curves. Results: Crude median survival time was 30 months. Women survived longer than men (the median survival time for women was 40 and for men was 24 months). Age (<60 vs >60 years old with hazard ratio (HR) of 2.43, 95% CI 1.3-4.5), primary pathology report (carcinoid vs. others with HR 5.85 cm, 95% CI 2.4-14.3), tumor size cm (for 5-10, HR of 3.1, 95% CI 1.6 and for >10 HR of 8.2, 95% with 95% CI 3.1-21.9), and chemotherapy with single drug (taking vs. not taking with a HR 2.2, 95% CI 1.1-4.8) had significant effects on overall survival of patients. Conclusions: Survival time in patients with neuroendocrine carcinomas is related to demographics, clinical characteristics, tumor histology, and subtype specific treatment.

Neuroendocrine Tumor of Unknown Primary Accompanied with Stomach Adenocarcinoma

  • Kim, Ho-Yeun;Choi, Sung-Il;Kim, Young-Ho
    • Journal of Gastric Cancer
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    • 제11권4호
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    • pp.234-238
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    • 2011
  • A 67 year old male at a regular checkup underwent esophagogastroduodenoscopy. On performing esophagogastroduodenoscopy, a lesion about 1.2 cm depressed was noted at the gastric angle. The pathology of the biopsy specimen revealed a well-differentiated adenocarcinoma. On performing an abdominal computed tomography (CT) scan & positron emission tomography-computed tomography (PET-CT) scan, no definite evidence of gastric wall thickening or mass lesion was found. However, lymph node enlargement was found in the left gastric and prepancreatic spaces. This patient underwent laparoscopic assisted distal gastrectomy and D2 lymph node dissection. On final examination, it was found out that the tumor had invaded the mucosal layer. The lymph node was a metastasized large cell neuroendocrine carcinoma with an unknown primary site. The patient refused chemotherapy. He opted to undergo a close followup. At the postoperative month 27, he had a focal hypermetabolic lesion in the left lobe of the liver that suggested metastasis on PET-CT scan. He refused to undergo an operation. He underwent a radiofrequency ablation.

T1 직장 신경내분비종양의 치료 (Management of T1 Rectal NEN)

  • 김지현;남승주
    • Journal of Digestive Cancer Research
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    • 제7권2호
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    • pp.45-50
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    • 2019
  • Incidence of rectal neuroendocrine neoplasms (NEN) has increased tremendously over the decades due to disease awareness and widespread use of screening colonoscopy. Although NEN has been recognized as malignant disease, most rectal NENs are initially found as small mass confined to the submucosa, which can be removed completely through various endoscopic treatments with good prognosis. In this review, we summarize the treatment options focusing on localized T1 rectal NEN by comparing representative international guidelines and discuss current controversies on the management. We also discuss various resection techniques focusing on endoscopic resection.

Clinicopathologic Significance of Gastric Adenocarcinoma with Neuroendocrine Features

  • Kim, Jang-Jin;Kim, June-Young;Hur, Hoon;Cho, Yong-Kwan;Han, Sang-Uk
    • Journal of Gastric Cancer
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    • 제11권4호
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    • pp.195-199
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    • 2011
  • Purpose: Composite neuroendocrine-exocrine carcinomas are malignancies that have two distinct components residing within the same tumor: an adenocarcinomatous portion and a neuroendocrine portion. This is rare in gastric cancers; however, poorly differentiated adenocarcinomas can sometimes reveal evidence of neuroendocrine features (NEF) or be 'mixed endocrine and exocrine carcinomas'. This study aimed to review NEF in gastric adenocarcinoma and to evaluate its prognostic significance. Materials and Methods: We selected 29 patients who were diagnosed with gastric adenocarcinoma with NEF and received gastrectomies at the Department of Surgery, Ajou University Hospital between January 2001 and December 2009. We analyzed the clinicopathologic features of gastric cancer with NEF and the prognosis associated with such tumors. Results: The pathologic result with respect to TNM staging of the gastric cancers with NEF were as follows: 5 cases of T1, 5 cases of T2, 10 cases of T3, and 9 cases of T4. There were 7 cases of N0, 7 cases of N1, 8 cases of N2 and 7 cases of N3. The staging of patients with NEF was higher than that of patients without NEF. Especially tumor lymphovascular invasion rate was 82.8%. The overall survival of patients with gastric cancer characterized by NEF was 73.8 months. Conclusions: Positive NEF status might be correlated with clinicopathologic parameters such as a high stage and high frequency of regional lymph node metastasis.

간외담도에 발생한 신경내분비암종 1예 (Large Cell Neuroendocrine Carcinoma of the Extrahepatic Bile Duct)

  • 박지영;전태주
    • 대한소화기학회지
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    • 제72권6호
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    • pp.318-321
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    • 2018
  • Primary neuroendocrine tumors originating from the extrahepatic bile duct are rare. Among these tumors, large cell neuroendocrine carcinomas (NECs) are extremely rare. A 59-year-old man was admitted to Sanggye Paik Hospital with jaundice that started 10 days previously. He had a history of laparoscopic cholecystectomy, which he had undergone 12 years previously due to chronic calculous cholecystitis. Laboratory data showed abnormally elevated levels of total bilirubin 15.3 mg/dL (normal 0.2-1.2 mg/dL), AST 200 IU (normal 0-40 IU), ALT 390 IU (normal 0-40 IU), and gamma-glutamyl transferase 1,288 U/L (normal 0-60 U/L). Serum CEA was normal, but CA 19-9 was elevated 5,863 U/mL (normal 0-37 U/mL). Abdominal CT revealed a 4.5 cm sized mass involving the common bile duct and liver hilum and dilatation of both intrahepatic ducts. Percutaneous transhepatic drainage in the left hepatic duct was performed for preoperative biliary drainage. The patient underwent radical common bile duct and Roux-en-Y hepaticojejunostomy for histopathological diagnosis and surgical excision. On histopathological examination, the tumor exhibited large cell NEC (mitotic index >20/10 high-power field, Ki-67 index >20%, CD56 [+], synaptophysin [+], chromogranin [+]). Adjuvant concurrent chemotherapy and radiotherapy were started because the tumor had invaded the proximal resection margin. No recurrence was detected at 10 months by follow-up CT.